Dr KARISSA LA BURNIY, Associate Professor GOPAL BASU, Dr HOLLY HUTTON
Biography:
Karissa La Burniy is a Renal Advanced Trainee at Alfred Health with a strong interest in glomerular diseases and transplant nephrology. She is actively involved in clinical research and case reporting, particularly in complex post-transplant outcomes. Her presentation on refractory pulmonary disease in MPO-ANCA vasculitis relapse post-renal transplant contributes to ongoing discussions about improving long-term outcomes in kidney health across Aotearoa and Australia.
Background:
Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a small-vessel vasculitis that can relapse despite immunosuppression. While transplant immunosuppression is presumed to offer protection against relapse, post-transplant recurrence remains a clinical concern.
Case Report:
We describe a patient diagnosed in 2014 with renal biopsy-confirmed myeloperoxidase (MPO) positive AAV without pulmonary involvement. Initial treatment with oral cyclophosphamide and corticosteroids achieved remission; however, multiple biopsy-confirmed renal relapses occurred between 2015 and 2018 following transition to azathioprine maintenance therapy. The disease progressed to end-stage renal failure, requiring haemodialysis from January 2019 after which all immunosuppression was ceased.
Relapse with pulmonary haemorrhage occurred in May 2020 and June 2021, managed with plasma exchange, corticosteroids, and rituximab. Remission was maintained on 6-monthly rituximab until renal transplantation in January 2024. MPO titres was weakly positive, with no clinical disease activity for 12 months prior to transplantation.
Transplant immunosuppression included basiliximab induction, tacrolimus, prednisone, and mycophenolate. This was adjusted to prednisone, leflunomide (then cyclosporine) and everolimus in the context of BK nephropathy and acute T cell-mediated rejection.
Despite therapeutic levels of immunosuppression, the patient experienced pulmonary vasculitis relapses in September 2024 and December 2024. Pulmonary haemorrhage was confirmed by radiology and bronchoscopy; infectious causes were excluded. Notably, MPO titres were within normal limits at relapse (0.8 IU/mL and 0.7 IU/mL respectively). C-reactive protein correlated with clinical flares and guided disease monitoring. The first relapse was managed with plasma exchange, corticosteroids and rituximab; the second with corticosteroids and obinutuzumab.
Conclusion:
This case underscores the unpredictable and relapsing nature of MPO-AAV, even under transplant immunosuppression. Normal MPO titres and isolated pulmonary involvement highlight the importance of vigilant monitoring and individualised post-transplant management.